【摘要】 目的回顾分析肝脏穿刺病理确诊为自身免疫性肝病(AILD)的人群的自身抗体及生化指标的特点,为我国AILD的筛查及诊断提供实验室依据。方法对首都医科大学附属北京地坛医院2008年7月至2011年6月经肝脏组织病理学诊断的65例AILD患者,采用描述性分析方法分析其自身抗体及生化指标。结果自身免疫性肝炎(AIH)39例,占60%;原发性胆汁性肝硬化(PBC)14例,占21.5%;原发性硬化性胆管炎(PSC)5例,占7.7%;PBC-AIH重叠综合征7例,占10.8%。中年女性多见。生化指标发现AIH中,ALT、AST、TBA、TBiL、GLO、GGT、ALP、IgG、RA、γ球蛋白高于正常值的比例分布于54%～100%。39例AIH患者中ANA阳性比例较高,为87.2%(34/39),SMA的阳性比例较低,为10.3%(4/39),无抗微粒体肝肾抗体(LKM)阳性病例。39例AIH患者中,有9例未查,30例AIH中Ro-52、SS-A、SS-B、抗nRNP/Sm抗体(nRNP/Sm)、CENPB、抗双链DNA抗体(dsDNA)、抗组蛋白抗体(Histones)的阳性比例分别为26.7%(8/30)、13.3%(4/30)、13.3%(4/30)、3.3%(1/30)、6.7%(2/30)、3.3%(1/30)、6.7%(2/30)。14例PBC患者中,2例未查,12例PBC中AMA、AMA-M2的阳性比例分别为50%(6/12)、41.7%(5/12);Ro-52、CENPB、抗核糖体P蛋白抗体(Rib.P.Protein)阳性比例分别为25%(3/12)、25%(3/12)、8.3%(1/12)。结论对于原因不明的慢性肝病患者,生化指标ALT、AST、TBA、TBiL、GLO、GGT、ALP、IgG、RA、γ球蛋白多项异常,要高度警惕AILD。非特异性抗体Ro-52、CENPB、SS-A阳性在AILD中占一定比例。ALP升高,即使自身抗体阴性也可以考虑AILD;TBA、TBiL升高,即使AMA-M2抗体阴性的患者也不能排除PBC;PBC-AIH重叠综合征中ANA、AMA、AMA-M2的阳性比例显著。对上述患者应早期行肝脏穿刺病理协助诊断。更多还原

【Abstract】 Objective To analyze antibodies and biochemical characteristics of patients finally diagnosed as autoimmune liver diseases(AILD)by liver biopsy,aim to provide laboratory evidence for screening and diagnosis of AILD in China.Methods The clinical data were collected and analyzed,including history of previous liver disease,accompanying symptoms,extrahepatic manifestations,biochemical tests,γ-glutamyl transferase(GGT),globulin(GLO),γglobulin,erythrocyte sedimentation rate,autoimmune antibodies,ENA spectra,of patients who were diagnosed as AILD by histopathology from July 2008 to June 2011 in Beijing Ditan Hospital affiliated to Capital Medical University.Results 65 patients were diagnosed as AILD by histopathology,including 39 cases autoimmune hepatitis(AIH),14 cases primary biliary cirrhosis(PBC),5 cases primary sclerosing cholangitis(PSC),7 cases PBC-AIH overlap syndrome.AILD was more common in middle-aged women:of 65 cases,14 cases were male,and 51 cases(78.46%)female,and 47 cases(72.30%)were beyond 40 years old.In autoimmune liver disease,the positive rates of alanine aminotransferase(ALT),aspartate aminotransferase(AST),γ-glutamyl transferase(GGT)were high,more than 80%.In PBC,anti-mitochondrial M2 subtype antibody(AMA-M2)-positive accounted for 41.7%.In PBC-AIH overlap syndrome,the rates of alkaline phosphatase(ALP)>2×ULN,GGT>5×ULN,ALT≥5×ULN were 71.4%(5/7),57.1%(4/7),14.3%(1/7)respectively,and positive rates of anti-nuclear antibody(ANA),anti-mitochondrial antibody(AMA),AMA-M2 were 100 %(7/7),71.4%(5/7),71.4%(5/7)respectively.The positive rates of non-specific antibody anti-Ro-52 antibody(Ro-52)in AIH,PBC,PBC-AIH overlap syndrome were 26.7%(8/30),25%(3/12),83.3%(5/6)respectively.The positive rates of anti-centromere protein B antibody(CENPB)in AIH,PBC,PBC-AIH overlap syndrome were 6.7%(2/30),25%(3/12),16.7%(1/6)respectively.The positive rates of anti-SS-A antibody(SS-A)in the AIH,BPC-AIH overlap syndrome were 13.3%(4/30),16.7%(1/6)respectively.Conclusions For patients with chronic liver disease of unknown cause,even if autoantibodies were negative,AILD should also be considered;for patients with negative AMA-M2 antibody,diagnosis of PBC could not be ruled out;non-specific antibodies Ro-52,CENPB,SS-A,etc accounted for a certain percentage in AILD;the positive rates of ANA,AMA,AMA-M2 in PBC-AIH overlap syndrome were predominant.更多还原